A Case of Adolescent Cronkhite-Canada Syndrome

Faria, Mikaell Alexandre Gouvêa; Basaglia, Bruna; Nogueira, Vinicius Quintiliano Moutinho; Mendonca, Tatiana Barros Gama Ferraz de; Kaiser, Roberto Luiz; Zotarelli-Filho, Idiberto José; Quadros, Luiz Gustavo de

doi:10.14740/gr912w

Cited by 8 publications

(5 citation statements)

References 5 publications

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“…The previous study with large sample size has reported that males and people aged 50–60 were the high-risk group [5‒7]. There were also a few sporadic cases reported among young people [9, 10]. Our findings from Chinese CCS patients were very similar with those results from other areas.…”

Section: Discussionsupporting

confidence: 88%

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Huang

Wang

et al. 2021

Dig Dis

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Introduction: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: 1) In Chinese population, people aged 50-70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. 2) The diverse range of GI manifestations were observed in all the patients, and almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%) . 4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). There is 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these GI polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients. Key words: Cronkhite-Canada syndrome, Clinical characteristics, Endoscopy

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Section: Discussionsupporting

confidence: 88%

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Huang

Wang

et al. 2021

Dig Dis

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“…[ 21 ] In this case, the patient’s clinical symptoms improved after 3 months of application of trace elements. CCS is characterized by the presence of diffuse gastrointestinal polyps, and multiple diffuse gastrointestinal polyps often involve the entire gastrointestinal tract outside the esophagus, [ 22 ] It is most commonly found in the stomach and colon, followed by the small intestine and rectum. Most of the gastric polyps and colonic polyps are sessile, mucosal congestion, and rare punctate bleeding.…”

Section: Discussionmentioning

confidence: 99%

“…Compared with juvenile polyposis syndrome, CCS polyps have fewer pedicles, inflammatory cell infiltration and edema in the lamina propria, and polyps have mucous abnormalities. [ 22 ] In addition, juvenile polyposis syndrome does not have epidermal features is also a differential point. [ 26 ] Despite the high rate of coincidence between CCS with gastrointestinal and colorectal cancers, it is unclear whether CCS is precancerous or whether it is associated with progression of conventional adenoma-carcinoma sequences.…”

Section: Discussionmentioning

confidence: 99%

Comprehensive treatment of Cronkhite-Canada syndrome: A case report and literature review

Hu¹,

Wu²,

Zhang

et al. 2023

Medicine

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Introduction: Cronkhite-Canada syndrome (CCS) is currently considered to be a non-hereditary disease, which is relatively rare clinically. It is also known as polyposis hyperpigmentation alopecia nail dystrophy syndrome, it is a syndrome characterized by gastrointestinal polyposis and ectodermal changes, the main manifestations are gastrointestinal symptoms, skin pigmentation, alopecia, and hypothyroidism. Case presentation: In this paper, the clinical characteristics, diagnosis and treatment of a case of CCS admitted to Huanghe Sanmenxia Hospital were analyzed. In the course of treatment, traditional Chinese medicine was used, but no hormone, and the patient’s clinical symptoms were greatly relieved. Conclusions: CCS is rare, there is no specific treatment, and traditional Chinese medicine may can greatly relieve the clinical symptoms of patients. However, it’s still having to be verified by a large sample, multi-center, long-term treatment follow-up studies.

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“…Protein-losing enteropathy is often observed in CCS because of polypoid lesions in the GI tracts. The mean age of initial diagnosis of CCS is between 50 and 60 years [3] and young patients have rarely been reported [4, 5].…”

Section: Discussion/conclusionmentioning

confidence: 99%

Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms

Murata¹,

Sato²,

Okada³

et al. 2020

Case Rep Gastroenterol

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Cronkhite-Canada syndrome (CCS) is a rare disease characterized by diffuse gastrointestinal polyposis with chronic diarrhea and ectodermal change, but its etiology is unknown. We present a case at the age of 26 years complaining of epigastralgia and weight loss. Endoscopic examination revealed extensive diffuse polypoid lesions of the stomach and the terminal ileum, all of which showed hyperplastic polyps pathologically. There were no polypoid lesions in his colon. He has no family history of diffuse gastrointestinal polyposis. Diffuse gastrointestinal hyperplastic polyposis without any hereditary association led us to suspect this case as CCS although he did not show chronic diarrhea and any ectodermal symptoms such as onychodystrophy, alopecia, and hyperpigmentation. After initiation of a corticosteroid therapy, his epigastralgia disappeared and he gained appetite and weight, accompanied by normalization of serum albumin levels. Endoscopic examination 1 year after initiation of corticosteroid therapy revealed a decrease in the number of gastric polyposis and those inflammations. This rare young case may suggest that early therapeutic intervention with corticosteroids could improve the prognosis of CCS, preventing not only malnutrition but also appearance of several ectodermal symptoms.

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A Case of Adolescent Cronkhite-Canada Syndrome

Cited by 8 publications

References 5 publications

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Comprehensive treatment of Cronkhite-Canada syndrome: A case report and literature review

Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms

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